Sodium vanadate combined with l-ascorbic acid delays disease progression, enhances motor performance, and ameliorates muscle atrophy and weakness in mice with spinal muscular atrophy
1 Institute of Molecular Biology, Academia Sinica, Taipei 115, Taiwan
2 Graduate Institute of Life Science, National Defense Medical Center, Taipei 114, Taiwan
3 Department of Neurology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei 100, Taiwan
4 Department of Life Science, National Taiwan Normal University, Taipei 116, Taiwan
BMC Medicine 2013, 11:38 doi:10.1186/1741-7015-11-38Published: 14 February 2013
Additional file 1:
Type II spinal muscular atrophy (SMA) patient-derived human dermal fibroblasts (HDFs) exhibit decreased survival motor neuron (SMN) levels. (A) Western blot analysis of HDF samples from a wild-type (WT) and SMA patient. β-Actin was used as an internal control. (B) Quantitation of the western blot results in (A). At least three independent experiments were carried out and the mean ± SEM was calculated. ***P < 0.001, t test.
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Additional file 2:
L-Ascorbic acid (L-AA) reduces sodium vanadate (SV)-induced SMN2-NSC34 cell death. Quantification of the viability of SMN2-NSC34 cells (Figure 1E) treated with L-AA, SV or SV combined with L-AA.
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Additional file 3:
L-Ascorbic acid (L-AA) reduces sodium vanadate (SV)-induced wild-type human dermal fibroblasts (WT-HDF) death. Quantification of the viability of WT-HDFs (Figure 1F) treated with L-AA, SV or SV combined with L-AA.
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Additional file 4:
L-Ascorbic acid (L-AA) reduces sodium vanadate (SV)-induced spinal muscular atrophy human dermal fibroblast (SMA-HDF) death. Quantification of the viability of SMA-HDFs (Figure 1G) treated with L-AA, SV or SV combined with L-AA.
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Additional file 5:
Determination of the lethal dose of combined treatment in type III spinal muscular atrophy (SMA) mice. The survival rates of mice that received L-ascorbic acid (L-AA), sodium vanadate (SV) alone and combined treatment were determined.
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Additional file 6:
Combined treatment does not improve motor function at an early age in type III spinal muscular atrophy (SMA) mice. (A-D) Motor functions were determined by surface righting assay (A), tube test (B) and negative geotaxis assay (C and D). Each group of mice showed no significant difference.
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